Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Feb 24;47(2):e20200166.
doi: 10.36416/1806-3756/e20200166. eCollection 2021.

Cystic fibrosis-related mortality trends in Brazil for the 1999-2017 period: a multiple-cause-of-death study

[Article in English, Portuguese]
Affiliations

Cystic fibrosis-related mortality trends in Brazil for the 1999-2017 period: a multiple-cause-of-death study

[Article in English, Portuguese]
Augusto Hasiak Santo et al. J Bras Pneumol. .

Abstract

Objective: To describe causes of death and mortality data related to cystic fibrosis (CF) using a multiple-cause-of-death methodology.

Methods: Annual mortality data for the 1999-2017 period were extracted from the Brazilian National Ministry of Health Mortality Database. All death certificates in which category E84 (CF) of the ICD-10, was listed as an underlying or associated cause of death were selected. Epidemiological and clinical data were described, and standardized mortality rates were calculated per year and for the 2000-2017 period. A joinpoint regression analysis was performed to detect changes in the mortality rates during the study period.

Results: Overall, 2,854 CF-related deaths were identified during the study period, ranging from 68 in 1999 to 289 in 2017. CF was the underlying cause of death in 83.5% of the death certificates. A continuous upward trend in the death rates was observed, with a significant annual percent change of 6.84% (5.3-8.4%) among males and 7.50% (6.6-8.4%) among females. The median age at death increased from 7.5 years in 1999 to 56.5 years in 2017. Diseases of the respiratory system accounted for 77% of the associated causes in the death certificates that reported CF as the underlying cause of death.

Conclusions: A significant and continuous increase in CF-related death rates was found in Brazil in the last years, as well as a concurrent increase in the median age at death.

Objetivo:: Descrever as causas de morte e dados sobre mortalidade relacionada à fibrose cística (FC) por meio da metodologia de causas múltiplas de morte.

Métodos:: Dados sobre a mortalidade anual no período de 1999 a 2017 foram extraídos do Sistema de Informações sobre Mortalidade do Ministério da Saúde do Brasil. Foram selecionadas todas as declarações de óbito em que a categoria E84 (FC) da CID-10 foi citada como causa básica ou associada de morte. Foram descritos os dados epidemiológicos e clínicos e calculadas as taxas padronizadas de mortalidade por ano e para o período de 2000 a 2017. Foi realizada a análise de regressão por pontos de inflexão para detectar mudanças nas taxas de mortalidade durante o período estudado.

Resultados:: No total, foram identificadas 2.854 mortes relacionadas à FC durante o período de estudo: de 68 em 1999 a 289 em 2017. A FC foi a causa básica de morte em 83,5% das declarações de óbito. Observou-se uma tendência contínua de aumento das taxas de mortalidade, com variação percentual anual significativa de 6,84% (5,3-8,4%) nos homens e de 7,50% (6,6-8,4%) nas mulheres. A mediana da idade de óbito aumentou de 7,5 anos em 1999 para 56,5 anos em 2017. As doenças do aparelho respiratório representaram 77% das causas associadas nas declarações de óbito em que a FC foi a causa básica de morte.

Conclusões:: Observou-se no Brasil um aumento significativo e contínuo das taxas de mortalidade relacionada à FC nos últimos anos, bem como um aumento concomitante da mediana da idade de óbito.

PubMed Disclaimer

Figures

Figure 1
Figure 1. Medians and interquartile ranges of age at death related to cystic fibrosis according to the regions in Brazil, 1999-2007.
Figure 2
Figure 2. Age-standardized death rates related to cystic fibrosis according to sex. Brazil, 2000-2017.
Figure 3
Figure 3. Trends in median age at death related to cystic fibrosis according to sex. Brazil, 1999-2017.

Comment in

  • Cystic fibrosis in Brazil: achievements in survival.
    Vendrusculo FM, Donadio MVF, Pinto LA. Vendrusculo FM, et al. J Bras Pneumol. 2021 Apr 30;47(2):e20210140. doi: 10.36416/1806-3756/e20210140. J Bras Pneumol. 2021. PMID: 33950101 Free PMC article. No abstract available.

References

    1. Ratjen F, Bell SC, Rowe SM, Goss CH, Quittner AL, Bush A. Cystic fibrosis. Nat Rev Dis Primers. 2015;1:15010–15010. doi: 10.1038/nrdp.2015.10. - DOI - PMC - PubMed
    1. O'Sullivan BP, Freedman SD. Cystic fibrosis. Lancet. 2009;373(9678):1891–1904. doi: 10.1016/S0140-6736(09)60327-5. - DOI - PubMed
    1. Raskin S, Pereira-Ferrari L, Reis FC, Abreu F, Marostica P, Rozov T. Incidence of cystic fibrosis in five different states of Brazil as determined by screening of p F508del, mutation at the CFTR gene in newborns and patients. J Cyst Fibros. 2008;7(1):15–22. doi: 10.1016/j.jcf.2007.03.006. - DOI - PubMed
    1. Brasil. Presidência da República. Casa Civil Lei no. 6.015, de 31 de dezembro de 1973. http://www.planalto.gov.br/ccivil_03/leis/l6015compilada.htm
    1. Brasil. Ministério da Saúde . Sistema de Informações de Mortalidade: Manual de Procedimentos Operacionais. Brasília: o Ministério; 1999.

LinkOut - more resources